Search PubMed⌕ Search

Biomedical subjects

Y Goldhammer

Publications and source records attributed to Y Goldhammer.

At least 37 records · Page 2Linked to original sources

Occurrence of both neurofibromatoses 1 and 2 in the same individual with a rapidly progressive course.

We describe a family in which the father had neurofibromatosis-1 and the mother neurofibromatosis-2. Their son presented at the age of 8 years with bilateral acoustic neuromas, meningioma, and numerous neurofibromas. We believe that the occurrence of the genes responsible for both forms of neurofibromatosis in the same patient had a synergistic effect on the early rapid growth of neurofibromatoses 1 and 2 neoplasms.

Adult↗

Vocal cord paralysis as a presenting sign in the Shy-Drager syndrome.

A 70-year-old patient with bilateral vocal cord paralysis, accompanied by inspiratory stridor and increased snoring, is presented. Respiratory dysfunction, deteriorating over 18 months, necessitated permanent tracheostomy. Only two and a half years after the onset of symptoms the patient developed extrapyramidal signs combined with severe autonomic failure, suggesting a diagnosis of Shy-Drager syndrome. Vocal cord palsy preceding any other neurological or autonomic manifestations of that syndrome has been infrequently described. This diagnosis should be considered in cases of vocal cord palsy of undetermined etiology, especially when associated with increased snoring or episodes of sleep apnea.

Aged↗

Sixth nerve palsy and unilateral Horner's syndrome.

Anatomic reports have demonstrated that the sympathetic fibers leave the carotid plexus to join the abducens nerve in the posterior part of the cavernous sinus. A lesion in this area may cause both an abducens nerve palsy and ipsilateral Horner's syndrome. This article details two additional cases of this uncommon occurrence.

Brain Neoplasms↗

Paroxysmal atrial fibrillation associated with an attack of multiple sclerosis.

A young woman presented with an acute right pontine lesion and paroxysmal atrial fibrillation. The lesion was later proven by magnetic resonance imaging to be due to multiple sclerosis. To our knowledge, cardiac arrhythmias have not been previously described in this condition. Published support for this possible association is reviewed.

Adult↗

Does prolonged use of diphenylhydantoin predispose to pulmonary sarcoidosis?

We describe 3 patients with epilepsy who developed sarcoidosis, stage I, while being treated with diphenylhydantoin for prolonged periods. We are unaware of such an association in the literature. However, in the light of the current knowledge about the adverse immunologic reactions that may be caused by diphenylhydantoin and the altered immunological state described in sarcoidosis, we recommend physicians to look for and report on similar cases in order to assess possible cause and effect relationships between the two.

Adult↗

Adrenoleukodystrophy in Israel: a genetic, clinical and biochemical study.

Adrenoleukodystrophy (ALD) is a fatal X-linked recessive lipid storage disease characterized by progressive CNS demyelination and adrenal insufficiency. Adrenomyeloneuropathy (AMN) is a variant of ALD, with a later onset and more prolonged course, presenting as a peripheral myeloneuropathy. A wide spectrum of clinical manifestations exists in both forms of the ALD complex. Affected infants are clinically normal at birth and in early infancy. Progressive cerebral dysfunction and adrenal failure appear usually between 5 and 10 years of age. Brain white matter macrophages, adrenal cortical cells and other tissues contain characteristic cytoplasmic inclusions. The specific biochemical abnormality in the ALD complex is an accumulation of very long-chain fatty acids (VLCFA) in different tissues and plasma, mainly tetracosanoic (C24:0) and hexacosanoic (C26:0) acids. Metabolic studies have been consistent with an oxidative defect of VLCFA. Clinical, genetic and biochemical data are presented on the first six families with documented ALD in Israel. There appears to be no ethnic predilection. ALD and AMN are found concomitantly, and all clinical forms are present.

Adolescent↗

Postictal blindness in adults.

Cortical blindness following grand mal seizures occurred in five adult patients. The causes of seizures included idiopathic epilepsy, vascular accident, brain cyst, acute encephalitis and chronic encephalitis. Blindness was permanent in one patients, but the others recovered within several days. Since most of the patients were either unaware of or denied their blindness, it is possible that this event often goes unrecognised. Cerebral hypoxia is considered the most likely mechanism.

Adult↗

Prolactinomas in women treated in the past for infertility.

This is a retrospective study on 352 infertile women who were treated with HMG-hCG (Human Menopausal Gonadotropin/human Chorionic Gonadotropin) between 1962 and 1977. The purpose of this study was to search for hyperprolactinemia and prolactinomas in these women. Hyperprolactinemia was found in 75 women (21%). The prevalence of hyperprolactinemia increases with age. Asymptomatic pituitary tumors were found in 29 of 31 women evaluated (94%). Three women had macroadenomas and 26 had microadenomas. We were not able to demonstrate that the HMG-hCG treatment and the ensuing pregnancy had any long-term ill effects on the size of the tumor. These findings suggest that prolactinomas were slow growing. An active search for these tumors must be made in women treated for infertility in the past.

Adenoma↗

Basal encephalocele associated with suprasellar epidermoid cyst.

A 27-year-old woman suffered from multiple congenital defects, including transsphenoidal encephalocele. Recent progressive visual loss was at first attributed to this encephalocele, but was later proved to be caused by a suprasellar epidermoid cyst. Its removal was followed by improvement of vision. To the best of our knowledge, the association of basal encephalocele and epidermoid cyst has not been previously described.

Abnormalities, Multiple↗

Intracranial esthesioneuroblastoma associated with unilateral visual loss. Case report.

Esthesioneuroblastoma is a rare tumor that arises from the olfactory mucosa and presents usually as a mass in the nasal cavity. Neurological complications occur in about 20% of these cases. Nine cases have been recorded so far in which the neoplasm manifested initially as an intracranial mass. These cases are reviewed and another patient, presenting with progressive unilateral visual loss, is reported.

Adolescent↗